Chest CT texture analysis for response assessment in systemic sclerosis.

PURPOSE: To evaluate the role of CT-textural features for monitoring lung involvement in subjects with systemic sclerosis(SSc) undergoing autologous stem cell transplantation(SCT) by comparison with semi-quantitative chest-CT, pulmonary function tests(PFT) and clinical parameters (modified Rodnan skin score[mRSS]). METHODS: In a retrospective single centre analysis, we identified 23 consecutive subjects(11male) with SSc between 07/2005 and 11/2016 that underwent chest CTs before, 6 and 12 months post-SCT. Response to therapy was defined at 6 months after transplantation as stabilisation or improvement in FVC > 10% and DLCOSB > 10%. CT-texture analysis(CTTA) including mean, entropy and uniformity were calculated. RESULTS: PFT classified the subjects into responders(18/23;78%) and non-responders(5/23;22%). mRSS improved in responders from 28.46 ±â€¯9.53 to 15.70 ±â€¯10.07 6 months after auto-SCT(p = .001) whereas in non-responders no significant improvement was registered. Fibrosis score increased significantly(mean2.33 ±â€¯1.23 vs.1.41 ±â€¯0.78; p = .005) in non-responders after 6 and 12months. Significant lower mean intensity and entropy of skewness and higher uniformity of skewness was found in responders vs. non-responders at baseline. Significant changes in CTTA-parameters were found in both responders and non-responders at 6months and only in responders also at 12months post-SCT. CONCLUSIONS: Changes in CT-textural features after SCT are associated with visual CT evaluation of SSc-related lung abnormalities, but complementary provide information about therapy-induced, structural pulmonary changes.

[Varicella gastritis under immunosuppression : Case report of a woman after lung transplantation due to granulomatosis with polyangiitis]. / Varizella-Gastritis unter Immunsuppression : Kasuistik einer Patientin nach Lungentransplantation bei Granulomatose mit Polyangiitis.

A 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. The gastroscopy showed diffuse ulcerative gastric lesions. Tests for varicella zoster virus and Epstein-Barr virus via polymerase chain reactions (PCR) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis. Intravenous antiviral therapy with acyclovir was administered resulting in a normalization of all clinical symptoms, especially of abdominal pain and inflammation parameters.

Imaging diagnosis in relapsing polychondritis and correlation with clinical and serological data.

OBJECTIVE: We hypothesize that imaging findings from CT and MRI correlate better with clinical markers for assessment of disease activity in patients with the rare relapsing polychondritis (RPC) than with serological inflammatory markers. MATERIALS AND METHODS: Retrospective database search at our institution identified 28 patients (13 females; age 49.0 years±15.0 SD) with RP between September 2004 and March 2014. Institutional review board approval was obtained for this retrospective data analysis. All patients had clinically proven RPC with at least two episodes of active disease. Of those, 18 patients were examined with CT- and MRI and presented all morphologic features of RPC like bronchial/laryngeal/auricular cartilage thickness, contrast enhancement, increased T2-signal intensity. Imaging data was subsequently correlated with corresponding clinical symptoms like fever, dyspnea, stridor, uveitis, pain, hearing impairment as well as with acute-phase-inflammatory parameters like C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). RESULTS: The clinical parameters were in good agreement with imaging findings and clinical symptoms such as tracheal wall thickening and dyspnea (r =0.65 p=0.05), joint synovitis on MRI and a higher McAdam score (r=0.84 p<0.001). No correlations were found between inflammatory laboratory markers, imaging findings and clinical features. CONCLUSION: Imaging diagnosis in RPC using CT and/or MRI delivers information about the degree of disease activity that correlates better with clinical features than unspecific inflammatory laboratory markers. Additionally, clinically unapparent cartilage involvement can be assessed adding value to the clinical diagnosis and therapy planning in this rare disease.

[Relapsing polychondritis. Primary and follow-up diagnostics with (18)F-FDG-PET/CT]. / Rezidivierende Polychondritis. Primär- und Verlaufsdiagnostik mittels (18)F-FDG-PET/CT.

CASE REPORT: This article presents an overview of the typical clinical and imaging features of relapsing polychondritis (RP), a rare autoimmune disease, based on a case report CONCLUSION: Although the diagnosis is mainly established clinically, the use of (18)F fluorodeoxyglucose positron emission tomography-computed tomography ((18)F-FDG-PET/CT) has been proven to be a useful diagnostic tool to accurately determine the extent of inflammation throughout the body [corrected]. Furthermore (18)F-FDG-PET/CT provides a high sensitivity for detection of a relapse, especially when clinical and laboratory results are inconclusive. Additionally, (18)F-FDG-PET/CT helps to assess disease activity during the course of therapy.

[Behçet's disease]. / Morbus Behçet.

Behçet's disease is a systemic disorder with the histopathological correlate of leukocytoclastic vasculitis. Pathogenetically, besides a strong genetic component participation of the innate immune system and an autoinflammatory component are discussed. The disease is most common in countries along the former silk route but in Germany the disease is rare (prevalence approximately 0.6/100,000). Oral aphthous ulcers are the main symptom, followed by skin manifestations, genital ulcers and oligoarthritis of large joints. Severe manifestations, threatening quality of life and even life itself, are the gastrointestinal manifestations which often perforate, arterial, mainly pulmonary arterial aneurysms which cause life-threatening bleeding, CNS manifestations and ocular disease, which with occlusive retinal vasculitis often leads to blindness. For milder manifestations low-dose steroids and colchicine are used, for moderate manifestations such as arthritis or ocular disease not immediately threatening visual acuity, azathioprin or cyclosporin A are combined with steroids. For severe manifestations, interferon-alpha, TNF-antagonists or cytotoxic drugs are recommended. Interleukin 1 (IL-1) antagonists are currently being examined in clinical studies.

[Extraocular manifestations of Behcet's disease]. / Extraokuläre Manifestationen des Morbus Behçet.

Behcet's disease is a multisystem disorder with the histopathological correlate of leukocytoclastic vasculitis. The classification criteria for the disease include the presence of recurrent oral aphthous ulcers combined with at least two other manifestations, such as genital aphthous ulcers, skin manifestations (mostly erythema nodosa or pseudofolliculitis) and ocular manifestations (panuveitis or posterior uveitis with retinal vasculitis). A positive pathergy test is regarded as pathognomonic for the disease and a sterile papulopustule occurs after a sterile needle prick of the forearm. However, this test is positive in only 15% of the patients. The prognosis of Behcet's disease becomes unfavorable when vital organs are involved. This is the case for involvement of the central nervous system which occurs in 10% of patients, arterial and pulmonary arterial aneurysms and gastrointestinal involvement, which clinically and histopathologically is difficult to differentiate from inflammatory bowel disease but tends to perforate. Oligoarthritis, which occurs in approximately 50% of the patients, causes problems concerning the differential diagnosis from classical forms of spondyloarthritis. Behcet's disease is associated with HLA-B51 in 50-80% of the cases depending on the country of origin of the patient. The prognosis becomes unfavorable if the disease manifests in young male patients. The treatment of extraocular manifestations depends on the aggressiveness. Milder manifestations are treated with low dose prednisolone and steroid sparing immunosuppressants, such as azathioprine or cyclosporine A. In cases with more severe manifestations, such as central nervous system (CNS) involvement cyclophosphamide or TNF antagonists and in selected cases also interferon alpha can be considered.